From the Guidelines
Symptoms to watch for in patients with suspected subacute sclerosing panencephalitis (SSPE) include progressive intellectual deterioration, behavioral changes, myoclonic jerks, seizures, and visual disturbances. The disease typically begins with subtle cognitive decline and personality changes that may initially be mistaken for behavioral problems, especially in children and adolescents 1. As SSPE advances, patients develop characteristic periodic myoclonic jerks that may be accompanied by EEG abnormalities showing periodic complexes, such as bilateral synchronous periodic sharp and slow waves 1. Motor dysfunction progressively worsens, with patients developing spasticity, ataxia, and eventually reaching a vegetative state with autonomic instability. Visual symptoms like cortical blindness and retinitis may occur. The disease follows a relentless course, typically progressing through four clinical stages from behavioral changes to coma. SSPE should be suspected in any young person with progressive neurological deterioration, especially with a history of measles infection or inadequate vaccination. Some key features to look out for include:
- Progressive intellectual deterioration
- Behavioral changes
- Myoclonic jerks
- Seizures
- Visual disturbances
- Characteristic EEG abnormalities It is essential to consider SSPE in the differential diagnosis of any young person with progressive neurological deterioration, given its rare but devastating consequences as a late complication of measles virus infection 1.
From the Research
Symptoms of Subacute Sclerosing Panencephalitis
The symptoms of subacute sclerosing panencephalitis (SSPE) can vary, but common manifestations include:
- Behavioral changes and cognitive decline 2, 3, 4
- Myoclonic jerks 5, 2, 3, 6
- Seizures, which can be focal or generalized 5, 2, 3
- Abnormalities in vision, including necrotizing retinitis and cortical blindness 2
- Bilateral pyramidal signs and coma 2
- Ataxia and spasticity 3, 6
- Visual disturbances and incontinence 6
- Psychiatric manifestations, such as regression in cognitive functioning and behavior 5, 4
Onset and Progression of Symptoms
The onset of SSPE is often insidious, with symptoms occurring an average of 6-10 years after measles infection 3, 4. The clinical course is characterized by progressive cognitive decline and behavior changes, followed by the development of seizures, myoclonus, and other symptoms 3. In some cases, the disease can progress rapidly, leading to a vegetative state and eventually death 3, 4.
Diagnosis and Management
Diagnosis of SSPE is often difficult in the early stages, but can be supported by clinical, electroencephalographic, and cerebrospinal fluid findings 2, 4. Management of the disease includes seizure control and avoidance of secondary complications associated with progressive disability 4. While there is no known cure for SSPE, treatment with interferon, ribavirin, and isoprinosine may prolong survival to some extent 2, 4.